Diseases of the mucous membrane in case of hypo-and avitaminosis and kollahenosis. Diseases of oral mucosa under exogenous intoxications (mercurial, plumbeous, bismuthous stomatitis).
Hypovitaminosis is insufficiency of one or more essential vitamins
Vitamin A deficiency
Vitamin A deficiency is a lack of vitamin A in humans. It is common in poorer countries but rarely seen in more developed countries that are wealthier. Night blindness is one of the first signs of vitamin A deficiency. Xerophthalmia, keratomalacia, and complete blindness can also occur since Vitamin A has a major role in phototransduction. Approximately 250,000 to 500,000 malnourished children in the developing world go blind each year from a deficiency of vitamin A, approximately half of whom die within a year of becoming blind. The United Nations Special Session on Children in 2002 set the elimination of vitamin A deficiency by 2010. The prevalence of night blindness due to vitamin A deficiency is also high among pregnant women in many developing countries. Vitamin A deficiency also contributes to maternal mortality and other poor outcomes in pregnancy and lactation.
Vitamin A deficiency also diminishes the ability to fight infections. In countries where children are not immunized, infectious disease like measles have higher fatality rates. Even mild, subclinical deficiency can also be a problem, as it may increase children’s risk of developing respiratory and diarrheal infections, decrease growth rate, slow bone development, and decrease likelihood of survival from serious illness.
Vitamin A deficiency is estimated to affect approximately one third of children under the age of five around the world. It is estimated to claim the lives of 670,000 children under five annually. Approximately 250,000-500,000 children in developing countries become blind each year owing to vitamin A deficiency, with the highest prevalence in Southeast Asia and Africa.
Signs and symptoms
Most common cause of blindness in developing countries is vitamin A deficiency (VAD). The World Health Organization (WHO) estimates 13.8 million children to have some degree of visual loss related to VAD. Night blindness and its worsened condition, xerophthalmia, are markers of VAD, as VAD can also lead to impaired immune function, cancer, and birth defects. Collections of keratin in the conjunctiva, known as “Bitot’s Spots,” are also seen.
Night blindness is the difficulty for the eyes to adjust to dim light. Affected individuals are unable to distinguish images in low levels of illumination. People with night blindness have poor vision in the darkness, but see normally when adequate light is present.
VAD affects vision by inhibiting the production of rhodopsin, the eye pigment responsible for sensing low light situations. Rhodopsin is found in the retina and is composed of retinal (an active form of vitamin A) and opsin (a protein). Because the body cannot create retinal in sufficient amounts, a diet low in vitamin A will lead to a decreased amount of rhodopsin in the eye, as there is inadequate retinal to bind with opsin. Night blindness results.
Night blindness caused by VAD has been associated with the loss of goblet cells in the conjunctiva, a membrane covering the outer surface of the eye. Goblet cells are responsible for secretion of mucus, and their absence results in xerophthalmia, a condition where the eyes fail to produce tears. Dead epithelial and microbial cells accumulate on the conjunctiva and form debris that can lead to infection and possibly blindness.
Decreasing night blindness requires the improvement of vitamin A status in at risk populations. Supplements and fortification of food have been shown to be effective interventions. Supplement treatment for night blindness includes high doses of vitamin A (200,000 IU) in the form of retinyl palmitate to be taken by mouth, which is administered two to four times a year. Intramuscular injections are poorly absorbed and are ineffective in delivering sufficient bio-available vitamin A. Fortification of food with vitamin A is costly, but can be done in wheat, sugar, and milk. Households may circumvent expensive fortified food by altering dietary habits. Consumption of yellow-orange fruits and vegetables rich in carotenoids, specifically beta carotene, provides pro-vitamin A precursors that will prevent VAD related night blindness.
Oral symptoms:
1.) delayed wound healing,
2.) xerostomia,
3.) altered taste,
4.) candidiasis,
5.) decrease in formation of ameoloblasts and odontoblasts
Causes
The major cause is diets which include few animal sources of pre-formed vitamin A. Breast milk of a lactating mother with vitamin A deficiency contains little vitamin A, which provides a breast-fed child with too little vitamin A.
In addition to dietary problems, there are other causes of vitamin A deficiency. Iron deficiency can affect vitamin A uptake. Excess alcohol consumption can deplete vitamin A, and a stressed liver may be more susceptible to vitamin A toxicity. People who consume large amounts of alcohol should seek medical advice before taking vitamin A supplements. In general, people should also seek medical advice before taking vitamin A supplements if they have any condition associated with fat malabsorption such as pancreatitis, cystic fibrosis, tropical sprue & biliary obstruction.
Treatment
Treatment of vitamin A deficiency can be undertaken with both oral and injectable forms, generally as vitamin A palmitate.
As an oral form, the supplementation of vitamin A is effective for lowering the risk of morbidity, especially from severe diarrhea, and reducing mortality from measles and all-cause mortality. Studies have shown that vitamin A supplementation of children under five who are at risk of vitamin A deficiency can reduce all‐cause mortality by 23 per cent. Some countries where vitamin A deficiency is a public health problem address its elimination by including vitamin A supplements available in capsule form with National Immunization Days (NIDs) for polio eradication or measles. Vitamin A capsules cost about US$0.02. The capsules are easy to handle; they don’t need to be stored in a refrigerator or vaccine carrier. When the correct dosage is given, vitamin A is safe and has no negative effect on seroconversion rates for Oral Polio Vaccine or measles vaccine. However, because the benefit of vitamin A supplements is transient, childreeed them regularly every four to six months. Since NIDs provide only one dose per year, NIDs-linked vitamin A distribution must be complemented by other dose programs to maintain vitamin A in children. Maternal high supplementation benefits both mother and breast-fed infant: high dose vitamin A supplementation of the lactating mother in the first month postpartum can provide the breast-fed infant with an appropriate amount of vitamin A through breast milk. However, high-dose supplementation of pregnant women should be avoided because it can cause miscarriage and birth defects.
Food fortification is also useful for improving vitamin A deficiency. A variety of oily and dry forms of the retinol esters, retinyl acetates and retinyl palmitate are available for food fortification of vitamin A. Margarine and oil are the ideal food vehicles for vitamin A fortification. They protect vitamin A from oxidation during storage and prompt absorption of vitamin A. β-carotene and retinyl acetate or retinyl palmitate are used as a form of vitamin A for vitamin A fortification of fat-based foods. Fortification of sugar with retinyl palmitate as a form of vitamin A has been used extensively throughout Central America. Cereal flours, milk powder, and liquid milk are also used as food vehicles for vitamin A fortification. Genetic engineering is another method of food fortification, and this has been achieved with golden rice,but opposition to genetically modified foods has prevented its use as of July 2012.
Dietary diversification can also control vitamin A deficiency. Non-animal sources of vitamin A which contain pre-formed vitamin A account for greater than 80% of intake for most individuals in the developing world. The increase in consumption of vitamin A-rich foods of animal origin in addition to fruits and vegetables has beneficial effects on vitamin A deficiency. Researchers at the Agricultural Research Service have been able to identify genetic sequences in corn that are associated with higher levels of beta-carotene, the precursor to vitamin A. They found that breeders can cross certain variations of corn to produce a crop with an 18-fold increase in beta-carotene. Such advancements iutritional plant breeding could one day aid in the illnesses related to vitamin A deficiency in developing countries.
Fig. 1. Vitamin A in food
Hypovitaminosis B1
Action
Vitamin B1 is required for oxidative decarboxylation of keto acids (pyruvic and lactic), the synthesis of acetylcholine; it is involved in carbohydrate metabolism and associated energy, fat, protein, water-salt metabolism, has a regulating effect on the trophic and the nervous system.
With insufficient intake of thiamine pyruvic and lactic acid accumulates in the tissues, disrupted the synthesis of acetylcholine, resulting in deteriorating since the functions of several systems, primarily, the nervous, cardiovascular and digestive.
Thiamine enhances circulation and is involved in hemopoiesis. Thiamine optimizes cognitive activity and brain function. He has a positive effect of the level of energy, growth, normal appetite, ability to learn and is necessary for muscle tone of the digestive tract, stomach and heart. Thiamine acts as an antioxidant, protecting the body from the damaging effects of aging, alcohol and tobacco.
Symptoms of hypovitaminosis
At full avitaminosis, B1 develops the disease of beri-beri: the body is disturbed carbohydrate metabolism, and accumulate lactic and pyruvic acid. In this case, observed damage to the nervous system (polyneuritis, which may end in paralysis), the heart muscle (it lost the ability to effectively shrink the heart of the patient increases, quickens the pulse), gastrointestinal tract (decreased appetite; constipation appeared). Patients with a sharp depletion of the total, partial or widespread swelling.
Fig. 1 Beriberi symptoms.
The primary symptoms develop hypovitaminosis B1 are:
The nervous system:
increased irritability, a feeling of inner restlessness, tearfulness,
depression,
insomnia (occasionally resistant) memory loss,
numbness in hands and feet, pain itch
impairment of coordination,
chill at room temperature,
disruption of the brain increased mental and physical fatigue;
Wernicke-Korsakoff syndrome (peculiar to patients suffering from alcoholism).
From the digestive system:
loss of appetite,
feeling of heaviness or burning in the epigastric region,
nausea,
constipation,
diarrhea,
weight loss
enlargement of the liver.
In oral cavity:
1.)Glossitus
2.)Glossodynia
3.)inflamed bleeding gums,
4.)angular cheilosis
5.)xerostomia
6.)altered taste
7.)burning tongue
8.)stomatitis amd mucositis
Cardio-vascular system:
shortness of breath, even at low physical activity,
tachycardia,
hypotension,
acute cardiovascular failure (may develop in some cases, the absence of timely diagnosis and treatment purposes).
Deficiency of vitamin B1 in the blood serum occurs in 25% of AIDS patients. Chronic thiamine deficiency in AIDS patients leads to a variety of neurological symptoms, leading to anorexia and weight loss.
Statement
Hypo-and avitaminosis B1 (beriberi).
Dosages
In therapeutic use for thiamine and thiamine chloride, bromide. Both drugs have a weak characteristic odor of yeast.
Drugs administered orally (after eating) and parenteral.
Dose of thiamine bromide is usually used at higher doses than thiamine chloride: 1 mg of thiamine chloride corresponds to the activity of 1.29 mg of thiamine bromide.
Doses by ingestion of thiamine chloride for adults 0,01 g (10 mg) 1-3 times a day. Children under three years – 0,005 grams (5 mg) a day, 3-8 years – 3 times a day, a day older than eight years – to 0.01 g 1-3 times a day. Course of treatment – 30 days.
Malabsorption in the intestine and the need for rapid creation of high concentrations of vitamin B1 in the blood it is administered intramuscularly: adults 0,025-0,05 g thiamine chloride or 0,03-0,06 g thiamine bromide 1 per day for children – 0, 0125 g of thiamine chloride, or 0,015 grams of thiamine bromide. Treatment course – 10-30 injections.
Security
Thiamin is generally well tolerated. Subcutaneous injections are painful because of the low pH solutions.
In rare cases (usually with parenteral administration) may be an allergic reaction (itching, urticaria, angioedema). The most severe reactions can occur with intravenous thiamine.
Allergic reactions usually occur in women in menopause and those suffering from alcoholism.
Vitamin B1 is contraindicated in individuals with drug intolerance in history.
Signs of hypervitaminosis
An overdose of vitamin B1 increases the activity of acetylcholine, which plays an important role in the pathogenesis of allergy.
Long-term introduction of excessive doses of vitamin B1 can lead to discoordination enzyme systems of liver and fatty degeneration, impaired renal function.
Interaction
Not recommended simultaneous parenteral administration of vitamin B1 with pyridoxine (vitamin B6) and cyanocobalamin (vitamin B12), as well as with penicillin, streptomycin and nicotinic acid.
Sulfonamides, as well as alcohol-based products infringe the normal absorption of vitamin B1. An antagonist of thiamine is choline. Antibiotics, drugs containing sulfur, oral contraceptives, antacids may reduce the level of thiamine in the body. For transfer of thiamine to its active form requires magnesium.
Fig. 2. Vitamin B1 in food.
B2 (Riboflavin) deficiency
Ariboflavinosis is the medical condition caused by deficiency of riboflavin (vitamin B2). Ariboflavinosis is most often seen in association with protein-energy malnutrition, and also in cases of alcoholism.
It was originally known as pellagra sin pellagra (pellagra without pellagra), as it exhibits certain similarities to the niacin deficiency pellagra.
The United States Recommended Daily Allowance (RDA) for riboflavin ranges from 1.1 to 1.3 milligrams per day for healthy adults to as high as 1.6 mg/day for pregnant or nursing women.
Riboflavin is continuously excreted in the urine of healthy individuals, making deficiency relatively common when dietary intake is insufficient. However, riboflavin deficiency is always accompanied by deficiency of other vitamins.
A deficiency of riboflavin can be primary – poor vitamin sources in one’s daily diet – or secondary, which may be a result of conditions that affect absorption in the intestine, the body not being able to use the vitamin, or an increase in the excretion of the vitamin from the body.
So, the most common cause of riboflavin deficiency is an inadequate diet; thus, it occurs most frequently in populations consuming limited quantities of riboflavin-containing foods such as meats, eggs, milk, cheese, yogurt, leafy green vegetables and whole grains. Riboflavin deficiency can also occur in those with impaired liver function, which prevents proper use of the vitamin. Borderline riboflavin deficiency as a consequence of certain anti-retroviral medications has also been known to cause acute lactic acidosis. An animal model of riboflavin kinase deficiency has been identified.
The signs and symptoms
The signs and symptoms of riboflavin deficiency typically include sore throat with redness and swelling of the mouth and throat mucosa, cheilosis and angular stomatitis (cracking of the lips and corners of the mouth), glossitis (magenta tongue with atrophy), seborrheic dermatitis or pseudo-syphilis (moist, scaly skin particularly affecting the scrotum or labia majora and the nasolabial folds), and a decreased red blood cell count with normal cell size and hemoglobin content (normochromic normocytic anemia).
Riboflavin deficiency is usually found together with other nutrient deficiencies, particularly of the other water-soluble vitamins. Phototherapy to treat jaundice in infants can cause increased degradation of riboflavin, leading to deficiency if not monitored closely. Persons with chronic alcoholism can have impaired absorption of riboflavin and other vitamins such as thiamine (see Wernicke’s encephalopathy).
Studies of the Turkoman people of Iran, who have a significantly increased incidence of esophageal cancer, have shown some relationship between chronic riboflavin deficiency and the onset of esophageal malignancies.
One study of pregnant women has found that riboflavin-deficient women were 4.7 times more likely to develop preeclampsia, though the mechanism for this is not known.
The most common symptoms:
– Bloodshot eyes
– Sore tongue and lips
– Infection in the mouth and throat
– Extreme and unusual sensitivity to light
– Irritability in the eyes
– Chapped lips
Fig.1 Ariboflavinosis. Oral cavity symptoms.
Treatment
Treatment involves a diet which includes an adequate amount of riboflavin. In cases related to weakened liver function, intravenous supplementation may be required.
Fig. 2. Vitamin B2 in food.
Vitamin PP (B3) deficiency.
Niacin (also known as vitamin B3, nicotinic acid, or less commonly vitamin PP; archaic terms include pellagra-preventive and anti-dermatitis factor) is an organic compound with the formula C6H5NO2 and, depending on the definition used, one of the 40 to 80 essential human nutrients.
Niacin is one of five vitamins (when lacking in human diet) associated with a pandemic deficiency disease: niacin deficiency (pellagra), vitamin C deficiency (scurvy), thiamin deficiency (beriberi), vitamin D deficiency (rickets and osteomalacia), vitamin A deficiency (night blindness and other symptoms). Niacin has been used for over 50 years to increase levels of HDL in the blood and has been found to modestly decrease the risk of cardiovascular events in a number of controlled human trials.
At present, niacin deficiency is sometimes seen in developed countries, and it is usually apparent in conditions of poverty, malnutrition, and chronic alcoholism. It also tends to occur in areas where people eat maize (corn, the only grain low in digestible niacin) as a staple food. A special cooking technique called nixtamalization is needed to increase the bioavailability of niacin during maize meal/flour production.
Mild niacin deficiency has been shown to slow metabolism, causing decreased tolerance to cold.
Severe deficiency of niacin in the diet causes the disease pellagra, which is characterized by diarrhea, dermatitis, and dementia, as well as “Casal’s necklace” lesions on the lower neck, hyperpigmentation, thickening of the skin, inflammation of the mouth and tongue, digestive disturbances, amnesia, delirium, and eventually death, if left untreated. Common psychiatric symptoms of niacin deficiency include irritability, poor concentration, anxiety, fatigue, restlessness, apathy, and depression. Studies have indicated that, in patients with alcoholic pellagra, niacin deficiency may be an important factor influencing both the onset and severity of this condition. Patients with alcoholism typically experience increased intestinal permeability, leading to negative health outcomes.
Hartnup’s disease is a hereditary nutritional disorder resulting iiacin deficiency. This condition was first identified in the 1950s by the Hartnup family in London. It is due to a deficit in the intestines and kidneys, making it difficult for the body to break down and absorb dietary tryptophan. The resulting condition is similar to pellagra, including symptoms of red, scaly rash, and sensitivity to sunlight. Oral niacin is given as a treatment for this condition in doses ranging from 40–200 mg, with a good prognosis if identified and treated early. Niacin synthesis is also deficient in carcinoid syndrome, because of metabolic diversion of its precursor tryptophan to form serotonin.
Niacin Deficiency Symptoms
Symptoms of mild niacin deficiency include:
– Indigestion
– Fatigue
– canker sores
– vomiting
– depression
Severe deficiency, called pellagra, can cause symptoms related to the skin, digestive system, and nervous system. They include:
– thick, scaly pigmented rash on skin exposed to sunlight
– swollen mouth and bright red tongue
– vomiting and diarrhea
– headache
– apathy
– fatigue
– depression
– disorientation
– memory loss
If not treated, pellagra can lead to death.
Skin manifestation:
-At first there is reddened skin with superficial scaling in areas exposed to sunlight, heat & friction. This may resemble severe sunburn then gradually subsides leaving a dusky brown-red colouration .
-The rash is usually symmetrical with a clear edge between affected and unaffected skin and sometimes may be itching or burning sensation
-Other features sometimes present include cheilosis, glossitis, angular stomatitis, and oral or perianal sores.
Fig. 1. Face and oral cavity manifestation of pellagra.
Fig. 2. Skin manifestation of pellagra.
Fig. 3. Skin manifestation of pellagra.
Niacin Deficiency Treatments
The recommend daily allowance (RDA) for niacin is 16 milligrams per day for men and 14 milligrams per day for women. For most people, a diet rich iiacin will prevent niacin deficiency. Good sources of niacin include red meat, fish, poultry, fortified breads and cereals, and enriched pasta and peanuts.
Fig. 4. Vitamin B3 in food.
Vitamin B12 deficiency
or hypocobalaminemia is a low blood level of vitamin B12. It can cause permanent damage to nervous tissue if left untreated longer than 6 months. Vitamin B12 itself was discovered through investigation of pernicious anemia, which is an autoimmune disease that destroys parietal cells in the stomach that secrete intrinsic factor. Pernicious anemia, if left untreated, is usually fatal within three years.[citatioeeded] Once identified, however, the condition can be treated fairly easily, although it cannot be cured and ongoing treatment is required. Humans obtain almost all of their vitamin B12 from dietary means. Pernicious anemia is usually the result of insufficient secretion of intrinsic factor within the stomach. Other more subtle types of vitamin B12 deficiency have been elucidated, including the biochemical effects, over the course of time in significant numbers.
Causes of Vitamin B12 Deficiency
Vitamin B12 deficiency can have a number of possible causes. Typically it occurs in people whose digestive systems do not adequately absorb the vitamin from the foods they eat. This can be caused by:
Pernicious anemia, a condition in which there is a lack of a protein called intrinsic factor. The protein, which is made in the stomach, is necessary for vitamin B12 absorption.
Atrophic gastritis, a thinning of the stomach lining that affects up to 30% of people aged 50 and older.
Surgery in which part of the stomach and/or small intestine is removed.
Conditions affecting the small intestine, such as Crohn’s disease, celiac disease, bacterial growth, or a parasite.
Excessive alcohol consumption.
Autoimmune disorders, such as Graves’ disease or systemic lupus erythematosus
Long-term use of acid-reducing drugs.
Vitamin B12 deficiency can also occur in vegetarians, because the best food sources of the vitamin are animal products. Strict vegans (people who don’t eat any animal products, including meat, eggs, or milk) are at greatest risk. Vegetarians who eat eggs and milk products are also at risk, because, on average, they consume less than half the adult Recommended Dietary Allowance (RDA) of vitamin B12.
Babies born to mothers who are vegetarians may also be deficient in vitamin B12.
Symptoms of Vitamin B12 Deficiency
A deficiency of vitamin B12 can lead to vitamin B12 deficiency anemia. A mild deficiency may cause only mild, if any, symptoms. But as the anemia worsens it may causes symptoms such as:
weakness, tiredness or light-headedness
rapid heartbeat and breathing
pale skin
sore tongue
easy bruising or bleeding, including bleeding gums
stomach upset and weight loss
diarrhea or constipation
If the deficiency is not corrected, it can damage the nerve cells. If this happens, vitamin B12 deficiency effects may include:
tingling or numbness in fingers and toes
difficulty walking
mood changes or depression
memory loss, disorientation, and dementia
B12 deficiency in infants, if not detected and treated, can lead to severe and permanent damage to the nervous system. New mothers who follow a vegetarian diet should have their babies’ B12 levels checked by a doctor.
Fig. 1. Atrophic glossitis as one of the symptoms of B12 deficiency
Treatment
Hydroxocobalamin injection USP (1000 mcg/mL) is a clear red liquid solution of hydoxocobalamin which is available in a 30 mL brown glass multidose vial packaged in a paper box. Shown is 500 mcg B-12 (as 1/2 cc) drawn up in a 1/2 cc U-100 27 gauge x 1/2″ insulin syringe, as prepared for subcutaneous injection.
B12 can be supplemented in healthy subjects by oral pill; sublingual pill, liquid, or strip; intranasal spray; transdermal patch or by injection. B12 is available singly or in combination with other supplements. B12 supplements are available in forms including cyanocobalamin, hydroxocobalamin, methylcobalamin, and adenosylcobalamin (sometimes called “cobamamide” or “dibencozide”). Oral treatments involve giving 250 µg to 1 mg of B12 daily.
Vitamin B12 can be given as intramuscular or subcutaneous injections of hydroxycobalamin, methylcobalamin, or cyanocobalamin. Body stores (in the liver) are partly repleted with half a dozen injections in the first couple of weeks (full repletion of liver stores requires about 20 injections) and then maintenance with monthly injections throughout the life of the patient. Vitamin B12 can also be easily self-administered by injection by the patient, using the same fine-gauge needles and syringes used for self-administration of insulin.
B12 has traditionally been given parenterally (by injection) to ensure absorption. However, oral replacement is now an accepted route, as it has become increasingly appreciated that sufficient quantities of B12 are absorbed when large doses are given. This absorption does not rely on the presence of intrinsic factor or an intact ileum. Generally 1 to 2 mg daily is required as a large dose. By contrast, the typical Western diet contains 5–7 µg of B12 (Food and Drug Administration (FDA) Daily Value ). It has been appreciated since the 1960s that B12 deficiency in adults resulting from malabsorption (including loss of intrinsic factor) can be treated with oral B12 supplements when given in sufficient doses. When given in oral doses ranging from 0.1–2 mg daily, B12 can be absorbed in a pathway that does not require an intact ileum or intrinsic factor. In two studies, oral treatment with 2 mg per day was as effective as monthly 1 mg injections.
Hypokalemia, an excessively low potassium level in the blood, is anecdotally reported as a complication of vitamin B12 repletion after deficiency. Excessive quantities of potassium are used by newly growing and dividing hematopoietic cells, depleting circulating stores of the mineral.
Research has established the effectiveness of other routes of B12 administration, primarily intranasal and sublingual dosing, but neither has been proven to be superior to oral dosing; recommendations are based on a consumers individual circumstances. The sublingual route, in which B12 is absorbed under the tongue, is manufactured in a variety of forms, such as lozenges, pills, and lollipops. A 2003 study found no significant difference in absorption for serum levels from oral vs. sublingual delivery of 500 µg (micrograms) of cobalamin, although the study measured only serum levels as opposed to tissue levels, which is more reflective of B12 levels. Sublingual methods of replacement may be effective only because of the typically high doses (500 micrograms), which are swallowed, not because of placement of the tablet. As noted below, such very high doses of oral B12 may be effective as treatments, even if gastro-intestinal tract absorption is impaired by gastric atrophy (pernicious anemia).
Fig. 2. Vitamin B12 in food.
Hypovitaminosis C
The classic vitamin C deficiency disease is scurvy. Early signs of the disease are bleeding gums and bleeding under the skin, causing tiny pinpoint bruises. The deficiency can progress to the point that it causes poor wound healing, anemia, and impaired bone growth. The body normally stores about 1,500 mg of vitamin C at a time, and symptoms of a deficiency do not occur until the body pool is less than 300 mg. It would take several weeks on a diet containing no vitamin C for this drop to occur in an otherwise well-nourished person.
Since only 10 mg of vitamin C is needed daily to prevent scurvy, the disease is rarely seen today. Even without signs of scurvy, a low intake of vitamin C can compromise many body functions, including the ability to rid the body of cholesterol and the immune system’s ability to fight off infection and disease.
People who smoke and women who use oral contraceptives have lower thaormal blood levels of vitamin C. In light of these findings in smokers, the current RDAs raised the amount of vitamin C required for smokers. They may need as much as 100 percent more vitamin C in their diets than nonsmokers.
Pathogenesis
Ascorbic acid is needed for a variety of biosynthetic pathways, by accelerating hydroxylation and amidation reactions. In the synthesis of collagen, ascorbic acid is required as a cofactor for prolyl hydroxylase and lysyl hydroxylase. These two enzymes are responsible for the hydroxylation of the proline and lysine amino acids in collagen. Hydroxyproline and hydroxylysine are important for stabilizing collagen by cross-linking the propeptides in collagen. Defective collagen fibrillogenesis impairs wound healing. Collagen is also an important part of bone, so bone formation is also affected. Defective connective tissue also leads to fragile capillaries, resulting in abnormal bleeding.
Symptoms
Early symptoms are malaise and lethargy. After 1–3 months, patients develop shortness of breath and bone pain. Myalgias may occur because of reduced carnitine production. Other symptoms include skin changes with roughness, easy bruising and petechiae, gum disease, loosening of teeth, poor wound healing, and emotional changes. Dry mouth and dry eyes similar to Sjögren’s syndrome may occur. In the late stages, jaundice, generalized edema, oliguria, neuropathy, fever, convulsions, and eventual death are frequently seen.
Oral symptoms:
1.)dellayed wound healing,
2.)inflamed bleeding gums
3.)inhibits fibroblasts, osteoblasts and odontoblasts
4.) teeth lost
5.) chronic forms of periodontal diseases
6.) stomatitis
7.) angular cheilitis
Fig. 1. Early symptoms of scurvy in oral cavity.
Fig. 2. Symptoms of scurvy in oral cavity.
Prevention
Scurvy can be prevented by a diet that includes certain citrus fruits such as oranges or lemons. Other sources rich in vitamin C are fruits such as blackcurrants, guava, kiwifruit, papaya, tomatoes, bell peppers, and strawberries. It can also be found in some vegetables, such as carrots, broccoli, potatoes, cabbage, spinach and paprika. Some fruits and vegetables not high in vitamin C may be pickled in lemon juice, which is high in vitamin C. Though redundant in the presence of a balanced diet, various nutritional supplements are available that provide ascorbic acid well in excess of that required to prevent scurvy, and even some candies and most soft drinks contain vitamin C as a preservative.
Many animal products, including liver, Muktuk (whale skin), oysters, and parts of the central nervous system, including the brain, spinal cord, and adrenal medulla, contain large amounts of vitamin C, and can even be used to treat scurvy.
Fresh meat from animals which make their own vitamin C (which most animals do) contains enough vitamin C to prevent scurvy, and even partly treat it. This caused confusion in the early history of scurvy, since the disease was only seen in people eating long-preserved diets or canned goods, but not in people eating any sort of fresh diet, including arctic diets primarily based upon meat. In some cases (notably in French soldiers eating fresh horse meat) it was discovered that meat alone, even partly cooked meat, could alleviate scurvy. In other cases, a meat only diet could cause scurvy. Some of these observations that scurvy was only associated with preserved foods prompted explorers to blame scurvy upon some type of tainting or poison which pervaded tinned foods.
Treatment
Scurvy can be treated by eating food containing vitamin C (such as oranges, papaya, strawberries, lemon), tablets with vitamin C, etc.
Prognosis
Untreated scurvy is invariably fatal. However, death from scurvy is rare in modern times. Since all that is required for a full recovery is the resumption of normal vitamin C intake, it is easy to treat if identified correctly. Consumption of dietary supplements and/or citrus fruits are means by which to accomplish this.
Fig. 3. Vitamin C in food.
Hypovitaminosis D
Hypovitaminosis D is a deficiency of vitamin D. It can result from inadequate nutritional intake of vitamin D coupled with inadequate sunlight exposure (in particular sunlight with adequate ultraviolet B rays), disorders that limit vitamin D absorption, and conditions that impair the conversion of vitamin D into active metabolites including certain liver, kidney, and hereditary disorders. Deficiency results in impaired bone mineralization and leads to bone softening diseases including rickets in children and osteomalacia and osteoporosis in adults.
Classifications:
Hypovitaminosis D is typically diagnosed by measuring the concentration in blood of the compound 25-hydroxyvitamin D (calcidiol), which is a precursor to the active form 1,25-dihydroxyvitamin D (calcitriol). One 2008 review has proposed the following four categories for hypovitaminosis D:
Insufficient 50–100 nmol/L (20–40 ng/mL)
Mild 25–50 nmol/L (10–20 ng/mL)
Moderate 12.5–25.0 nmol/L (5–10 ng/mL)
Severe < 12.5 nmol/L (< 5 ng/mL)
Signs and symptoms:
Vitamin D deficiency is known to cause several bone diseases including:
– Rickets, a childhood disease characterized by impeded growth, and deformity, of the long bones. The earliest sign of subclinical vitamin D deficiency is Craniotabes, abnormal softening or thinning of the skull. In oral cavity – enamel hypoplasia, occlusion defects, enlarged pulp chambers, dental abscess (fig. 1, 2).
– Osteomalacia, a bone-thinning disorder that occurs exclusively in adults and is characterized by proximal muscle weakness and bone fragility.
– Osteoporosis, a condition characterized by reduced bone mineral density and increased bone fragility.
– Muscle aches and weakness (in particular proximal limb girdle)
– Muscle twitching (Fasciculations)
The role of diet in the development of rickets was determined by Edward Mellanby between 1918–1920. In 1921 Elmer McCollum identified an anti-rachitic substance found in certain fats that could prevent rickets. Because the newly discovered substance was the fourth vitamin identified, it was called vitamin D. The 1928 Nobel Prize in Chemistry was awarded to Adolf Windaus, who discovered the steroid 7-dehydrocholesterol, the precursor of vitamin D.
Prior to the fortification of milk products with vitamin D, rickets was a major public health problem. In the United States, milk has been fortified with 10 micrograms (400 IU) of vitamin D per quart since the 1930s, leading to a dramatic decline in the number of rickets cases.
Hypovitaminosis D is also considered a risk factor for the development of depressive symptoms in older persons.
Radiograph of a two-year old rickets sufferer, with a marked genu varum (bowing of the femurs) and decreased bone opacity, suggesting poor bone mineralization.
Risk factors:
Age
The amount of vitamin D recommended for all infants, children, and adolescents has recently increased – from 400 to 600 IU per day. The Institute of Medicine released the Consensus Report on Dietary Reference Intakes for Calcium and Vitamin D on November 30, 2010. IOM recommends 600 IU of vitamin D a day for those 1-70 and 800 IU for those over 70 years of age. As of October 2008, the American Pediatric Association advises vitamin D supplementation of 400 IU/day (10μg/d) from birth onwards. (1 IU Vitamin D is the biological equivalent of 0.025 μg cholecalciferol/ergocalciferol.) The daily dose of 400 IU is required to prevent rickets and possibly also a wide range of chronic nonskeletal diseases.The Canadian Paediatric Society recommends that pregnant or breastfeeding women consider taking 2000 IU/day, that all babies who are exclusively breastfed receive a supplement of 400 IU/day, and that babies living above 55 degrees latitude get 800 IU/day from October to April. Health Canada recommends 400IU/day (10μg/d). Infant formula is generally fortified with vitamin D.
Malnutrition
Although rickets and osteomalacia are now rare in many countries, there have been outbreaks in some immigrant communities in which osteomalacia sufferers included women with seemingly adequate daylight outdoor exposure wearing Western clothing. Having darker skin and reduced exposure to sunshine did not produce rickets unless the diet deviated from a Western omnivore pattern characterized by high intakes of meat, fish and eggs, and low intakes of high-extraction cereals. The dietary risk factors for rickets include abstaining from animal foods. Vitamin D deficiency remains the main cause of rickets among young infants in most countries, because breast milk is low in vitamin D and social customs and climatic conditions can prevent adequate UVB exposure. In sunny countries such as Nigeria, South Africa, and Bangladesh where the disease occurs among older toddlers and children it has been attributed to low dietary calcium intakes, which are characteristic of cereal-based diets with limited access to dairy products. Rickets was formerly a major public health problem among the US population; in Denver where ultraviolet rays are approximately 20% stronger than at sea level on the same latitude almost two thirds of 500 children had mild rickets in the late 1920s. An increase in the proportion of animal protein in the 20th-century American diet coupled with increased consumption of milk fortified with relatively small quantities of vitamin D coincided with a dramatic decline in the number of rickets cases.
Sun exposure
The use of sunscreen with a sun protection factor (SPF) of 8 can theoretically inhibit more than 95% of vitamin D production in the skin. In practice, however, sunscreen is applied so as to have a negligible effect on vitamin D status. The vitamin D status of those in Australia and New Zealand is unlikely to have been affected by campaigns advocating sunscreen. Instead, wearing clothing is more effective at reducing the amount of skin exposed to UVB and reducing natural vitamin D synthesis.
Another risk factor arising from lack of sun exposure is clothing which covers a large portion of the skin. This clothing when worn on a consistent and regular basis, such as the burqa, is correlated with lower vitamin D levels and an increased prevalence of hypovitaminosis D.
Darker skin color
It has been suggested the reduced pigmentation of light-skinned individuals results in higher vitamin D levels and that, because melanin acts like a sun-block, dark-skinned individuals, in particular, may require extra vitamin D to avoid deficiency at higher latitudes. The natural selection hypothesis suggests that lighter skin color evolved to optimise vitamin D production in extreme northern and southern latitudes.
Rickets is sometimes due to genetic disorders such as autosomal dominant hypophosphatemic rickets or X-linked hypophosphatemia and associated with consanguineous marriage, and possibly founder effect. In Kashmir, India patients with pseudovitamin D deficiency rickets had grossly raised 25-hydroxyvitamin D concentrations. Skin colour has also been associated with low 25(OH)D, especially in Africans living in countries with a temperate climate. For example 25-OHD under 10 ng/mL (25 nmol/l) in 44% of asymptomatic East African children living in Melbourne. However a study of healthy young Ethiopians living in Addis Ababa (10 degrees N) found average 25(OH)D levels of 23.5nmol/L. A review of vitamin D in Africa gives the median levels for equatorial countries: Kenya 65.5 nmol/L and Democratic Republic of the Congo 65nmol/L, concluding that it remains to be established if associations between vitamin D status and health outcomes identified in Western countries can be replicated in African countries.
Vitamin D levels are approximately 30% higher in northern Europe than in central and southern Europe; higher vitamin D concentrations iorthern countries may have a genetic basis. In a meta-analysis of cross-sectional studies on serum 25(OH)D concentrations globally the levels averaged 54 nmol/l and were higher in women than men, and higher in Caucasians than ion-Caucasians. There was no trend in serum 25(OH)D level with latitude. African Americans often have a very low circulating 25(OH)D level. However, those of African descent have higher parathyroid hormone and 1,25-Dihydroxycholecalciferol associated with lower 25-hydroxyvitamin D than other ethnic groups; moreover, they have the greatest bone density and lowest risk of fragility fractures compared to other populations. Deficiency results in impaired bone mineralization, and leads to bone softening diseases.
Diagnosis
The serum concentration of 25-hydroxy-vitamin D is typically used to determine vitamin D status. It reflects vitamin D produced in the skin as well as that acquired from the diet, and has a fairly long circulating half-life of 15 days. It does not, however, reveal the amount of vitamin D stored in other body tissues. The level of serum 1,25-dihydroxy-vitamin D is not usually used to determine vitamin D status because it has a short half-life of 15 hours and is tightly regulated by parathyroid hormone, calcium, and phosphate, such that it does not decrease significantly until vitamin D deficiency is already well advanced.
One study found that vitamin D3 raised 25-hydroxy-vitamin D blood levels more than did vitamin D2, but this difference has been adequately disproved to allow reasonable assumption that D2 and D3 are equal for maintaining 25-hydroxy-vitamin D status.
There has been variability in results of laboratory analyses of the level of 25-hydroxy-vitamin D. Falsely low or high values have been obtained depending on the particular test or laboratory used. Beginning in July 2009 a standard reference material became available which should allow laboratories to standardise their procedures.
There is some disagreement concerning the exact levels of 25-hydroxy-vitamin D needed for good health. A level lower than 10 ng/mL (25 nmol/L) is associated with the most severe deficiency diseases: rickets in infants and children, and osteomalacia in adults. A concentration above 15 ng/ml (37.5 nmol/L) is generally considered adequate for those in good health. Levels above 30 ng/ml (75 nmol/L) are proposed by some as desirable for achieving optimum health, but there is not yet enough evidence to support this.
Levels of 25-hydroxy-vitamin D that are consistently above 200 ng/mL (500 nmol/L) are thought to be potentially toxic, although data from humans are sparse. In animal studies levels up to 400 ng/mL (1,000 nmol/L) were not associated with toxicity. Vitamin D toxicity usually results from taking supplements in excess. Hypercalcemia is typically the cause of symptoms, and levels of 25-hydroxy-vitamin D above 150 ng/mL (375 nmol/L) are usually found, although in some cases 25-hydroxy-vitamin D levels may appear to be normal. It is recommended to periodically measure serum calcium in individuals receiving large doses of vitamin D.
In overweight persons increased fat mass is inversely associated with 25(OH)D levels. This association may confound the reported relationships between low vitamin D status and conditions which occur more commonly in obesity as the circulating 25(OH)D underestimates their total body stores. However, as vitamin D is fat-soluble, excess amounts can be stored in fat tissue and used during winter months, when sun exposure is limited.
A study of highly sun-exposed (tanned) healthy young skateboarders and surfers in Hawaii found levels below the proposed higher minimum of 30 ng/ml in 51% of the subjects. The highest 25(OH)D concentration was around 60 ng/ml (150nmol/L). A similar <using the same data>study in Hawaii found a range of (11–71 ng/mL) in a population with prolonged extensive skin exposure while as part of the same study Wisconsin breastfeeding mothers were given supplements. The range of circulating 25(OH)D levels in women in the supplementated group was from 12–77 ng/mL. It is noteworthy that the levels in the supplemented population in Wisconsin were higher than the sun exposed group in Hawaii (which again included surfers because it was the same data set).
Another study of African Americans found that blood levels of 25(OH)D decreased linearly with increasing African ancestry, the decrease being 2.5-2.75 nmol/L per 10% increase in African ancestry. Sunlight and diet were 46% less effective in raising these levels among subjects with high African ancestry than among those with low/medium African ancestry. It could be possible that vitamin-D metabolism differs by ethnicity.
Fig. 3. Vitamin D in food.
Systemic scleroderma
Systemic sclerosis or systemic scleroderma is an autoimmune or connective tissue disease. It is characterized by thickening of the skin caused by accumulation of collagen, and by injuries to the smallest arteries. There are two overlapping forms. Limited cutaneous scleroderma is limited to the skin on the face, hands and feet. Diffuse cutaneous scleroderma covers more of the skin, and is at risk of progressing to the visceral organs, including the kidneys, heart, lungs and gastrointestinal tract are affected.
Survival is determined by the severity of visceral disease. Prognosis is difficult to predict until the disease differentiates into recognizable subsets. Patients with limited cutaneous scleroderma have a good prognosis, with 10-year survival of 75%, although <10% develop pulmonary arterial hypertension after 10 to 20 years. Patients with diffuse cutaneous scleroderma have a 10-year survival of 55%. Death is most often from pulmonary, heart and kidney involvement, although survival has greatly improved with effective treatment for kidney failure. Immunosuppressive drugs are used, although glucocorticoids have limited application.
Signs and symptoms
Fig. 1. Scleroderma involving the hands. A, Edematous phase with diffuse swelling of fingers. B, Atrophic phase with contracture and thickening sclerodactyly (thick skin over the fingers).
Diffuse Scleroderma – affects the skin as well as the heart, lungs, GI tract, and kidneys.
Limited Scleroderma – mostly affects the skin of the face, neck and distal elbows and knees and late in the disease causes isolated pulmonary hypertension. CREST syndrome (Calcinosis, Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiectasias) is associated with limited scleroderma.
Skin symptoms
In the skin, systemic sclerosis causes hardening and scarring. The skin may appear tight, reddish or scaly. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage may weaken limbs and affect appearance. Also, patients report substantial, even severe and recurrent itching of large skin areas, the source of much affliction as the condition worsens. There is much variation in severity between patients, with some having scleroderma of only a limited area of the skin (such as the fingers) and little involvement of the underlying tissue; while others have progressive skin involvement.
Other organs
Diffuse scleroderma can cause musculoskeletal, pulmonary, gastrointestinal, renal and other complications.Patients with larger amounts of cutaneous involvement are more likely to have involvement of the internal tissues and organs. Most patients (over 80%) have vascular symptoms and Raynaud’s phenomenon, which leads to attacks of discoloration of the hands and feet in response to cold. Raynaud’s normally affects the fingers and toes. Systemic scleroderma and Raynaud’s can cause painful ulcers on the fingers or toes which are known as digital ulcers. Calcinosis (deposition of calcium in lumps under the skin) is also common in systemic scleroderma, and is often seen near the elbows, knees or other joints.
Musculoskeletal
The first joint symptoms that patients with scleroderma have are typically non specific joint pains, which can lead to arthritis, or cause discomfort in tendons or muscles. Joint mobility, especially of the small joints of the hand, may be restricted by calcinosis or skin thickening. Patients may develop muscle weakness, or myopathy, either from the disease, or its treatments.
Lungs
Some impairment in lung function is almost universally seen in patients with diffuse scleroderma on pulmonary function testing; however, it does not necessarily cause symptoms, such as shortness of breath. Some patients can develop pulmonary hypertension, or elevation in the pressures of the pulmonary arteries. This can be progressive, and lead to right sided heart failure. The earliest manifestation of this may be a decreased diffusion capacity on pulmonary function testing.
Other pulmonary complications in more advanced disease include aspiration pneumonia, pulmonary hemorrhage and pneumothorax.
Digestive tract
Endoscopic image of peptic stricture, or narrowing of the esophagus near the junction with the stomach due to chronic gastroesophageal reflux. This is the most common cause of dysphagia, or difficulty swallowing, in scleroderma.
Diffuse scleroderma can affect any part of the gastrointestinal tract. The most common manifestation in the esophagus is reflux esophagitis, which may be complicated by peptic stricturing, or benign narrowing of the esophagus. This is best initially treated with proton pump inhibitors for acid suppression, but may require bougie dilatation in the case of stricture.
Scleroderma can decrease motility anywhere in the gastrointestinal tract. The most common source of decreased motility involvement is the esophagus and the lower esophageal sphincter, leading to dysphagia and chest pain. As Scleroderma progresses, esophageal involvement from abnormalities in decreased motility may worsen due to progressive fibrosis (scarring). If this is left untreated, acid from the stomach can back up into the esophagus causing esophagitis, and GERD. Further scarring from acid damage to the lower esophagus many times leads to the development of fibrotic narrowing, also known as strictures which can be treated by dilatation, and Barrett’s esophagus. The small intestine can also become involved, leading to bacterial overgrowth and malabsorption, of bile salts, fats, carbohydrates, proteins, and vitamins. The colon can be involved, and can cause pseudo-obstruction or ischemic colitis.
Rarer complications include pneumatosis cystoides intestinalis, or gas pockets in the bowel wall, wide mouthed diverticula in the colon and esophagus, and liver fibrosis. Patients with severe gastrointestinal involvement can become profoundly malnourished.
Scleroderma may also be associated with gastric antral vascular ectasia (GAVE), also known as watermelon stomach. This is a condition where atypical blood vessels proliferate usually in a radially symmetric pattern around the pylorus of the stomach. GAVE can be a cause of upper gastrointestinal bleeding or iron deficiency anemia in patients with scleroderma.
Oral cavity signs
– pursed lips – dificult to open the mouth
– esophageal sclerosis – gastroesophageal reflux – damage of enamel
– pale, rigid mucosa
– teleangiectasias
– decreased mobility of tongue
– salivary hypofunction
Fig. 2. Oral manifestation of scleroderma
Scleroderma can have a significant adverse effect upon the health of the mouth. A wide variety of different problems can arise that may result in increased liability to dental decay (caries), gingivitis and difficulty with dentures. Furthermore these oral problems, in particular xerostomia (mouth dryness) and microstomia (limited mouth opening) can reduce the quality of life of affected individuals.
Xerostomia
Xerostomia (oral dryness) can be a complication of scleroderma, specifically in those individuals with secondary Sjogren’s syndrome (dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia) and a connective tissue disease, e.g. scleroderma and others). The oral dryness may also be worsened by the use of drugs such as antidepressants and antihypertensives which individually can also cause xerostomia. The resultant xerostomia causes dryness, and possible soreness, of the lining of the mouth (oral mucosa). In addition there is an increased liability to dental decay (caries), gum inflammation (gingivitis), possibly fungal infections (e.g. thrush) and loss of retention of the denture – particularly the upper denture. Patients with oral dryness may also have reduced or altered taste sensation, oral malodour (halitosis) and reduced quality of sleep.
Microstomia
Microstomia is probably the most significant oral consequence of scleroderma, giving rise to limited mouth opening, and as a result difficulty with eating and perhaps speech. The limited mouth opening can also make it difficult for affected individuals to insert and remove dentures and undergo routine dental care.
Other features
Some drugs used in the treatment of scleroderma may give rise to oral manifestations, for example gingival enlargement (e.g. calcium channel blockers). Telangiectasia (dilated blood vessels) can occur on the oral mucosa, lips and face. A number of skeletal changes about the face can also arise leading to loss of bone of the cheek bones (zygomatic arch) and lower jaw (mandible), although these are unlikely to give rise to symptoms. The skeletal changes are most likely in patients who have tightness of the facial skin.
Management of oral mucosal dryness
It is clearly important that patients avoid agents (for example alcohol and tobacco) that will worsen any existing oral dryness. Patients often attempt to substitute saliva by sipping water or non-sugary drinks. It is best, however, to avoid sugary agents as these will increase the risk of dental decay, and non-sugary drinks (e.g. fizzy drinks) give rise to mild chemical erosion of the outer surfaces of teeth.
A number of synthetic salivary substitutes are available as sprays and/or mouthwashes. These agents are mildly viscous, may contain fluoride and interestingly can be slightly acidic (and thus theoretically might increase the risk of dental erosion). There is no one particular salivary substitute that seems to be better than another – each patient has his or her own preference. Recently a mouthwash based upon an extract of linseed oil has been suggested to be of benefit in the treatment of xerostomia as it may reduce intra-oral plaque as well as lessen any xerostomia. There are, however, no studies of the effectiveness of this in patients with scleroderma.
A number of gels have been suggested to lessen oral dryness, in particular BioXtra® and Oral Balance®. These agents can be applied to any oral mucosal surface as often as the person so wishes, but again there is great variation as regards the benefit of this to each individual. It has, however, been suggested that Oral Balance® may reduce the burning sensation associated with oral dryness and also aid eating and swallowing. It may be possible to stimulate salivary flow. For example sucking sweets can cause some increase in salivary flow, but this increases the risk of dental decay. Diabetic sweets (which do not contain sucrose) can be helpful, but the sorbitol that is present in these products can cause gastrointestinal upset in some individuals. Chewing gum can be beneficial, but not all patients like this. Pilocarpine prescribed by doctors and dentists can specifically increase salivary function again, however, this agent has a number of adverse side effects, in particular gastrointestinal upset. Occasional patients receiving pilocarpine also report increased sweating.
As a consequence of the microstomia and oral dryness, some individuals with scleroderma find that their upper lip becomes adherent to the upper anterior teeth. It is possible to lessen this by applying a lubricating jelly (e.g. KY jelly®) to the inside of the lips and aspects of the upper teeth such that the lips glide over the teeth.
A wide variety of alternative agents have been suggested to be of benefit for the treatment of oral dryness associated with scleroderma and secondary Sjogren’s syndrome, these include evening primrose oil, anhydrous crystalline maltose and dehydroepiandrosterone (DHEA). In general these agents appear to cause a slight improvement in oral dryness – if at all.
The dryness of the mouth associated with scleroderma reflects immunological destruction of the gland. Unfortunately no immuno -suppressant regime has been found to be effective in lessening or indeed reversing the inflammatory destruction of the glandular tissue. It is possible for corticosteroids to be injected down the ducts of the major salivary glands to reduce, albeit transiently, the oral dryness. Unfortunately this procedure would have to be repeated and therefore is of little clinical application. Most recently it has been suggested that gene therapy in which a virus, modified to allow local expression of proteins to enhance salivary flow, is injected within the ductal tissue. At present this method is in the early stages of development.
Prevention of dental decay and gingivitis
Patients with scleroderma are at increased risk of dental decay and gum disease as a consequence of the oral dryness and difficulty of mouth opening. It is important to lessen the risk of long-term consequences of these oral diseases (e.g. abscesses, extractions, tooth mobility and loss of teeth). A number of simple measures should be considered:
Diet
It is important that all patients (with or without scleroderma) have a diet that avoids frequent and/or excess sticky/sweet foods. These foods increase the accumulation of dental plaque and in turn increase the risk of dental decay. Savoury foods are much less likely than sugary ones to cause dental decay.
Tooth cleaning
Teeth should be cleaned at least twice daily. It is of course difficult for individuals with scleroderma to clean their teeth as a consequence of the poor mouth opening and fibrous nature of the linings of the mouth. It would thus seem best to use a toothbrush which has a small head with soft nylon bristles as this will allow the toothbrush to clean all parts of mouth. Handles of conventional toothbrushes can be modified to enable patients with reduced manual dexterity to easily hold the brush. Advice on appropriate modifications can be obtained from a dentist, hygienist or therapist. Electric toothbrushes do allow teeth to be cleaned very effectively, particularly as they have a small head, but some of them are slightly heavy due to them containing a battery within the handle. Toothpaste that contains fluoride should be used as this hardens the outer surfaces of the teeth. Additionally fluoride mouthwash (e.g. Fluoraguard®) used weekly or, better still, daily help harden up the outer surface of the teeth. Fluoride tablets are not of notable benefit to adults.
Inter-dental cleaning
Toothbrushing only cleans the outer surfaces of the teeth. Fluorides do not give rise to any adverse side effects – provided of course they are used correctly. It is important, if possible, to clean between the teeth (interdentally). Inter-dental cleaning can be undertaken using floss, of which there are many varieties, dental brushes or wood sticks. Electric flossers (e.g. Oral B Hummingbird) are also available. Wood sticks and inter-dental brushes should only be used where there are spaces between the teeth, as forcing brushes or sticks may result in trauma to the gingiva.
Gingivitis is lessened by the aforementioned toothcleaning methods. In addition regular use of antimicrobial mouthwash that contains chlorhexidine, triclosan or any other similar antimicrobial further reduce the risk of gingivitis. Chlorhexidine can give rise to staining of the teeth and can have an unpleasant taste, although the former may be reduced by using the mouthwash immediately following toothcleaning.
Denture problems in scleroderma
Patients with microstomia can have difficulties in inserting and removing their dentures from the mouth. In addition the microstomia can make it difficult for impressions to be taken during the construction of dentures. These difficulties can be overcome by the construction of dentures that comprise 2 parts, thus allowing the appliance to easily pass into the mouth. Likewise impressions can be undertaken in sections.
Xerostomia can cause the upper denture to become easily dislodged. This can be lessened by placing synthetic saliva on the fitting surface of the denture. Osseo-integrated implants are a means of ensuring the retention of dentures. These are titanium screws that are placed within the jaw bones, the bone eventually uniting with the titanium of the implant. It is then possible to construct either dentures that clip onto the implant, or bridges that firmly attached to implants. There are no major contraindications to the placement of implants in patients with scleroderma, although it can be difficult to place implants in patients with severe microstomia. It must, however, be highlighted that implants are very expensive and Primary Care Trusts may be reluctant to fund such treatment.
References:
1. Danilevskiy M.F. et al. “ Diseases of the mucous membrane of the mouth.” – K.: “Medytsyna”, 2010.
2. http://en.wikipedia.org/wiki/Hypovitaminosis_D
3. http://things-to-know-about-health.blogspot.com/2011/05/vitamin-b1-thiamine.html
4. http://en.wikipedia.org/wiki/Ariboflavinosis
5. http://en.wikipedia.org/wiki/Vitamin_B12_deficiency
6. http://en.wikipedia.org/wiki/Scurvy
7. http://www.scleroderma.org.uk/scleroderma/scleroderma
Information was prepared by Sukhovolets I.O.
